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Cold Urticaria

Written by Dr Harris Steinman

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Cold urticaria is a disorder characterised by the rapid onset of itchiness, redness and swelling of the skin within minutes after exposure to a cold stimulus.

The sensation of burning may be a prominent feature. The swelling is limited to the parts of the body that have been in contact with the stimulus.

The symptoms are often worse after the exposed area is warmed.

Holding of cold objects can cause swelling of the hand. Eating cold food may result in swelling of the lips, but swelling of the tongue and throat occurs less frequently. Generalised symptoms of flushing, headache, fainting and abdominal pain may occur if large areas are affected.

Patients typically experience symptoms while outside on cold, windy or rainy days. Fatalities following swimming have been reported and those affected should be warned that swimming or having a cold bath could be dangerous.

Onset:
This disease can begin at any age, affects both sexes equally, and affects young adults most commonly.

Diagnosis:
If the condition is suspected, a simple test (Ice-cube test) can be performed.

Place an ice cube on the patient’s forearm for 4 minutes and observe the area for 10 minutes afterwards. If the patient has cold urticaria, the area will become itchy and then swell approximately 2 minutes after removing the ice cube.

Differential Diagnosis:
The most commonly form of cold urticaria seen in practise is idiopathic cold contact urticaria. There are other conditions in which exposure to cold results in whealing. (Table 1)

Table 1. Other causes of whealing from Cold Exposure

  • Cold-induced (reflex) cholinergic urticaria:
  • Familial (essential) cold urticaria
  • Cryoglobulinaemia
  • Cold agglutinin disease
  • Cryofibrinoganemia
  • Paroxysmal cold hemoglobinuria
  • Post infection.
  • Insect stings.

These other conditions are rare, and are associated with other symptoms. In these conditions the Ice-cube test is usually negative. Cold urticaria may follow an insect sting reaction or a virus infection such as Mycoplasma pneumonia.
 

Cold-induced (reflex) cholinergic urticaria results in generalised urticaria when patients are exposed to the cold, but they do not respond to a local stimulus. The severity of the symptoms increases with exercising in cold weather.
 

Familial (essential) cold urticaria is a rare form of cold intolerance that is inherited. It is not a true urticaria. A generalised reaction occurs approximately 30 minutes following exposure to the cold and consists of burning papular skin lesions, fever, chills, headaches, joint and muscle pain, and can last up to 48 hours.
 

Aquagenic urticaria (contact with water) may be confused with cold urticaria. This condition results from exposure to water of any temperature.
 

Management:
Treatment consists of patient education, stimulus avoidance and medication. (Table 2)

The newer antihistamines (H1 receptor blockers e.g. Zyrtec) may be very effective. H2 receptor blockers such as Tegamet may also be effective.
 

The induction of tolerance by repeated regional or generalised cold exposure has had variable results. This should be performed on an in-patient basis and would require a well-motivated patient.

Table 2. Management

  • Patient education
  • Avoid exposure to cold stimuli, including swimming or bathing in cold water.
  • Supply with easily injectable adrenaline e.g. Epipen, Ana-Guard or Medihaler-Epi inhaler
  • Enrol with Medic Alert
  • Optimise treatment of concurrent allergic conditions
  • Substitute Beta-Blockers with another suitable agent.

Outcome:
The severity of cold urticaria varies considerably. It may resolve spontaneously in a few months or last for 6-9 years or longer, but its course is usually very unpredictable.

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